scholarly journals Multiple aplasia cutis congenita type V and fetus papyraceous: a case report and review of the literature

2021 ◽  
Vol 15 (1) ◽  
Author(s):  
V. Thadchanamoorthy ◽  
Kavinda Dayasiri ◽  
M. Thirukumar ◽  
N. Thamilvannan ◽  
S. H. Chandraratne
Keyword(s):  
Rare Condition ◽  
The Body ◽  
Sri Lankan ◽  
Aplasia Cutis Congenita ◽  
Skin Defects ◽  
Underlying Causes ◽  
Cutis Aplasia ◽  
Contracture Formation ◽  
Type V ◽  
Aplasia Cutis

Abstract Background Aplasia cutis congenita is regarded as congenital focal absence of skin in the newborn, and occurrence of more than three similar skin defects is rare. The etiology is thought to be multifactorial, and precise etiopathogenesis is unknown. Case presentation A 13-day-old newborn Sri Lankan Tamil girl was referred to the dermatologic clinic with multiple skin defects at birth. There were six lesions on the body, and two of them had healed during intrauterine period, leaving scars. This was a second twin of her pregnancy. Her first twin fetus had demised before 19 weeks of pregnancy and was confirmed to be fetus papyraceous based on ultrasound-guided fetal assessment. The said child was thoroughly investigated and found to have no other congenital abnormalities. Chromosomal studies yielded normal findings. She was treated with tropical antibacterial ointment, and all lesions resolved spontaneously within 4 weeks, leaving scars. Physiotherapy was commenced to prevent contracture formation, and follow-up was arranged in collaboration with the plastic surgical team. Conclusions Aplasia cutis congenita is a rare condition of uncertain etiology, but consanguinity may play a role. This report described a newborn with type V cutis aplasia congenita in whom the diagnosis was confirmed based on clinical features and revision of antenatal history. The management depends on the pattern, extent, location, severity, underlying causes, and associated anomalies.

scholarly journals Aplasia cutis congenita with fetus papyraceus: an uncommon case report

2020 ◽  
Vol 6 (6) ◽  
pp. 809
Author(s):  
Vivekananda Ittigi ◽  
Aparna Anto
Keyword(s):  
Reproductive Technologies ◽  
In Utero ◽  
Multiple Gestation ◽  
Aplasia Cutis Congenita ◽  
Multiple Gestations ◽  
Symmetrical Distribution ◽  
Associated Conditions ◽  
Uncommon Case ◽  
Type V ◽  
Aplasia Cutis

<p class="abstract">Aplasia cutis congenita is a condition characterized by congenital absence of all skin layers in a localized or widespread area. Frieden's classification recognized a rare subtype, type V, that is associated with multiple gestations in which there is the in-utero demise of a twin with resultant fetus papyraceus or mummification. A twin pregnancy was complicated by in-utero death of one twin at 5 months. On full term birth of the other twin with fetus papyraceus large defects in the skin of knees were noted bilaterally. Conservative management with topical antibiotics and emollients helped in complete re-epithelialization in few months. As the use of reproductive technologies increase the incidence of multiple gestation and associated conditions are expected to increase. This type is clinically unique in that it is characterized by stellate lesions in a symmetrical distribution over the trunk and extremities, differing from other subtypes, which are typically localized to scalp (70%-85% of cases) or extremities. Management ranges from conservative to surgical grafts.</p>

scholarly journals Type V aplasia cutis congenita

2010 ◽  
Vol 30 (2) ◽  
pp. 171-172 ◽  
Author(s):  
Umar A. Qureshi ◽  
Nisar Ahmed
Keyword(s):  
Aplasia Cutis Congenita ◽  
Type V ◽  
Aplasia Cutis

Multiple Aplasia Cutis Congenita Lesions of the Scalp: A Case Study

2020 ◽  
Vol 39 (2) ◽  
pp. 83-91
Author(s):  
Mary Whalen
Keyword(s):  
Chromosomal Abnormalities ◽  
Rare Condition ◽  
Trisomy 13 ◽  
Aplasia Cutis Congenita ◽  
Live Births ◽  
Patau Syndrome ◽  
Males And Females ◽  
Aplasia Cutis

Aplasia cutis congenita (ACC) is a rare condition that presents at birth as an absence of skin that does not usually involve underlying structures. Occurring in 3/10,000 live births, ACC is evenly distributed between males and females; the risk of ACC increases to 7 percent in consanguineous marriages. Up to 86 percent of lesions are found on the scalp in the midline vertex position. Lesions can also be found on the trunk and limbs, as with Adams-Oliver syndrome or accompanying epidermolysis bullosa. ACC is associated with chromosomal abnormalities and 35–50 percent of the time with trisomy 13 (Patau syndrome). This case study presents an infant with multiple ACC lesions of the scalp. The pathophysiology, treatment, potential long-term complications, and nursing considerations are discussed.

Type V Aplasia Cutis Congenita: Case Report, Review of the Literature, and Proposed Treatment Algorithm

2012 ◽  
Vol 30 (6) ◽  
pp. e208-e213 ◽  
Author(s):  
Daniel Morrow ◽  
Robert Schelonka ◽  
Alfons Krol ◽  
Michael Davies ◽  
Anna Kuang
Keyword(s):  
Case Report ◽  
Treatment Algorithm ◽  
Review Of The Literature ◽  
Aplasia Cutis Congenita ◽  
Type V ◽  
Aplasia Cutis

scholarly journals A rare case report of aplasia cutis congenita of the scalp with triplet skin defects in non-syndromic newborn

2021 ◽  
Vol 8 (3) ◽  
pp. 997
Author(s):  
Naim Sulaiman Abuzarifa ◽  
Wan Azman Wan Sulaiman
Keyword(s):  
Case Report ◽  
Gestational Age ◽  
Rare Case ◽  
Surgical Intervention ◽  
Skin Condition ◽  
Skin Defect ◽  
Aplasia Cutis Congenita ◽  
Skin Defects ◽  
Rare Case Report ◽  
Aplasia Cutis

Aplasia cutis congenita is an uncommon localized or widespread congenital skin condition characterized by the absence of the skin, and occasionally underlying tissues occurs in about one every 10.000 birth mostly in the scalp as single or more than one lesion and sometimes occurs in extremities and trunk with an uncertain cause and can be associated with numerous syndromes or can be sporadic which is diagnosed clinically and usually conservatively managed but sometimes surgical intervention needed. In this literature, we present nonsyndromic newborn Malay girl normally delivered with 35 weeks gestational age with triplet skin raw areas at the vertex of the scalp with well-demarcated defect round in shape measured about 0.5 in radius covered with a noninflammatory, necrotic patch. To our knowledge, many works of literatures presented Aplasia cutis congenita at the scalp with solitary single or occasionally more than one skin defect. In contrast, in our case, we present an infrequent rare case of triplet skin defect of aplasia cutis congenita for nonsyndromic newborns. 

scholarly journals Aplasia Cutis Congenita. Case report and literature review

2019 ◽  
Vol 30 (3) ◽  
pp. 267-272
Author(s):  
Moysés Isaac Cohen ◽  
Jefferson Travessa Ribeiro ◽  
Cleinaldo De Almeida Costa ◽  
Fernando Luís Westphal
Keyword(s):  
Subcutaneous Tissue ◽  
Bone Lesion ◽  
Treatment Strategies ◽  
Rare Condition ◽  
Aplasia Cutis Congenita ◽  
Skull Defects ◽  
Increased Risk ◽  
Csf Leakage ◽  
Multiple Treatments ◽  
Aplasia Cutis

Aplasia Cutis Congenita (ACC) is a rare condition characterized by congenital absence of epidermis, dermis and, in some cases, like this one, subcutaneous tissue and bone. It usually involves the scalp vertex. The estimated incidence is 3 in 10,000 births resulting in a total number of 500 reported cases till nowadays. The lesion can occur on everybody surface, but scalp is the most affected region (70% of the cases). In about 20% of cases there is bone lesion. ACC can occur as an isolated defect or can be associated with a number of other congenitally anomalies such as limb anomalies or embryologic malformations. In patients with large scalp and skull defects, there is increased risk of infection and bleeding along with increased mortality and therefore prompt and effective management is advised. Case presentation: We describe a child with ACC involving almost all skull, where it could be seen the brain only with arachnoid membrane. At presentation there was no Cerebral Spinal Fluid (CSF) leakage. And it was managed surgically. The child was operated on one time. It was covered with non-absorbable matrix and the skin was approached. Conclusion: There are multiple treatments for this condition. But there is no consensus on treatment strategies. Conservative treatment can be tried, especially when there is no bone lesion. The treatment must be individualized for each case.

scholarly journals A Case of Aplasia Cutis Congenita with Widespread Multifocal Skin Defects Without Extracutaneous Abnormalities

2019 ◽  
Vol 99 (3) ◽  
pp. 343-344 ◽  
Author(s):  
J Kim ◽  
J Kim ◽  
M Lee ◽  
E Huh ◽  
J Lim ◽  
...  
Keyword(s):  
Aplasia Cutis Congenita ◽  
Skin Defects ◽  
Aplasia Cutis

Cutis Aplasia: Perioperative Management and Case Report

2012 ◽  
Vol 21 (3) ◽  
pp. 212-215
Author(s):  
Steven M. Levine ◽  
Derek D. Reformat ◽  
Charles H. Thorne
Keyword(s):  
Perioperative Management ◽  
Wound Care ◽  
Surgical Techniques ◽  
Full Thickness ◽  
Aplasia Cutis Congenita ◽  
Rare Congenital Disorder ◽  
Scalp Defect ◽  
Life Threatening ◽  
Cutis Aplasia ◽  
Aplasia Cutis

Aplasia cutis congenita, a rare congenital disorder involving defects of some or all of the layers of the cranium, is associated with potential life-threatening complications. Although treatment involves both nonsurgical and surgical techniques, the importance of perioperative management cannot be overstressed. A multidisciplinary team, including personnel from nursing, neonatology, pediatrics, radiology, neurosurgery, and plastic surgery services, diagnosed aplasia cutis congenita and planned local wound care, surgical correction, and prevention of potentially life-threatening complications in a 1-day-old boy with a 6×5-cm full-thickness scalp defect.

scholarly journals Aplasia Cutis Congenita: A Case Report

2018 ◽  
Vol 10 (2) ◽  
pp. 182-186 ◽  
Author(s):  
Tahani Magliah ◽  
Faiza Alghamdi
Keyword(s):  
Case Report ◽  
Positive Family History ◽  
Rare Condition ◽  
Medical Intervention ◽  
Intracranial Extension ◽  
Congenital Defects ◽  
Anterior Fontanel ◽  
Aplasia Cutis Congenita ◽  
Scalp Lesion ◽  
Aplasia Cutis

Background: Aplasia cutis congenita is characterized by congenital focal absence of skin in a newborn. Case Report: A 45-day-old boy presented to the outpatient clinic of dermatology at King Abdul Aziz Medical City, Jeddah, Saudi Arabia, with a flat scalp lesion on the space of the anterior fontanel. There was a positive family history of such condition in his older brother’s scalp, which resolved spontaneously within 3 weeks after labor, without any medical intervention. There were no associated hemangiomata or other congenital defects in his body. The case was admitted to the hospital. Ultrasound of the head showed bullae over the anterior fontanel, well-defined complex cyst, and an isolated subcutaneous lesion, with no evidence of intracranial extension. The baby was started on intravenous infusion of vancomycin (67 mg in dextrose 5% in water) for 3 days, but no improvement occurred to the scalp cyst. The baby started to receive 15 g of 2% mupirocin ointment, topically three times daily. After 2 days, the scalp cyst gradually decreased in size and became dry within 1 week. Follow-up after 3 months showed that the scalp lesion completely healed, leaving a very small atrophic scar and no further management of the lesion was needed. Conclusions: Aplasia cutis congenita is a rare condition of uncertain etiology, but consanguinity may play a role. Its management depends on its pattern, location, underlying causes, and associated anomalies.

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